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Atypical cutaneous lymphoproliferative disorder in patients with HIV infection
Author(s) -
Friedler Suzanne,
Parisi Mary T..,
Waldo Elaine,
Wieczorek Rosemary,
Sidhu Gurdip,
Rico M. Joyce
Publication year - 1999
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1046/j.1365-4362.1999.00417.x
Subject(s) - medicine , mycosis fungoides , cutaneous lymphoma , lymphoma , pathology , dermatology , hypopigmentation , paraneoplastic pemphigus , cutaneous t cell lymphoma , biopsy , immunology , antibody , autoantibody
Background An inflammatory pruritic eruption which is characterized histologically by CD8+, atypical lymphocytes has been described in human immunodeficiency virus (HIV)‐infected patients. This disorder has been described as “pseudo‐Sezary” or a “cutaneous T cell lymphoma (CTCL)‐simulant”, however, as these patients do not resemble CTCL clinically, and the relationship between this entity and CTCL is unclear, a more descriptive term is “atypical cutaneous lymphoproliferative disorder” (ACLD). The purpose of this study is to categorize the clinical, histologic, and immunophenotypic features of 16 HIV‐infected patients with this skin disorder seen at the New York Department of Veterans Affairs Medical Center. Methods A retrospective chart review was conducted on 16 HIV‐infected patients with a histologic diagnosis of an atypical cutaneous lymphoproliferative infiltrate on skin biopsy. Skin biopsies were reviewed, and histologic features noted. Immunophenotyping was performed on 14 out of 16 samples; electron microscopy was performed on samples from five patients. Clinical manifestations, disease course, medication history, and response to treatment were noted. Results The patients presented with a pruritic, persistent, generalized, papular eruption. Pigment changes, including hyperpigmentation and hypopigmentation were common. Histologically, lesional biopsies were characterized by a superficial and deep polymorphous infiltrate with atypical lymphocytes which were CD8+ predominant, Ki‐1 negative, and occasionally CD7 antigen depleted. Sezary‐like cells were present in the infiltrate in four out of five patients by electron microscopy. None of the patients has systemic manifestations of lymphoma; however, one of the 16 patients eventually developed frank CTCL. Conclusions HIV‐infected patients can present with a pruritic, widespread disorder, often with pigment changes characterized by an atypical cutaneous lymphocytic infiltrate. This clinicopathologic disorder is a rare, reactive inflammatory condition which generally occurs in late stage HIV infection and rarely progresses to frank lymphoma.